Primary epithelioid angiosarcoma of the adrenal gland: aggressive histological features and clinical behavior

Primary adrenal epithelioid angiosarcoma (PAEA) is an exceedingly rare and high-grade malignant neoplasm with an epithelioid appearance morphologically. To the best of our knowledge, only 35 cases of PAEA have previously been reported in English literature. Here, we present our 7-year experience (2010-2017) with adrenal lesions at our Clinical Pathological Diagnosis Center. Of more than 1,800 adrenal gland specimens, a total of 2 adrenal epithelioid angiosarcoma cases were identified. We reviewed the literature for 13 similar cases. All of the patients appeared to have morphological features of epithelioid cells including vascular endothelium immunophenotype, hemorrhage/necrosis, and anastomosing vascular channels. They underwent adrenalectomy without chemotherapy or radiotherapy. One was still well at 3-year follow up but the other had died at 1-month follow up. One of our cases was first described with accompanying reactive adrenal cortex hyperplasia in a local area. Moreover, the mechanisms of epithelioid angiosarcoma are still uncertain and require further study. Clinical prognosis is poor, therefore early accurate diagnosis and complete resection of this tumor may be very helpful for patients.